Pelvic Ewing Sarcoma Research Articles

Prognostic factors and overall survival in pelvic Ewing's sarcoma and chordoma: A comparative SEER database analysis

BackgroundThis study aimed to develop and validate nomograms to predict overall survival (OS) for pelvic Ewing's sarcoma (EWS) and chordoma, identify prognostic factors, and compare outcomes between the two conditions. MethodsWe identified patients diagnosed with pelvic EWS or chordoma from the SEER database (2001–2019). Independent risk factors were identified using univariate and multivariate Cox regression analyses, and these factors were used to construct nomograms predicting 3-, 5-, and 10-year OS. Validation methods included AUC, calibration plots, C-index, and decision curve analysis (DCA). Kaplan-Meier curves and log-rank tests compared survival differences between low- and high-risk groups. ResultsThe study included 1175 patients (EWS: 611, chordoma: 564). Both groups were randomly divided into training (70 %) and validation (30 %) cohorts. OS was significantly higher for chordoma. Multivariate analysis showed year of diagnosis, income, stage, and surgery were significant for EWS survival, while age, time to treatment, stage, and surgery were significant for chordoma survival. Validation showed the nomograms had strong predictive performance and clinical utility. ConclusionsThe nomograms reliably predict overall survival (OS) in pelvic EWS and chordoma, helping to identify high-risk patients early and guide preventive measures. The study also found that survival rates are significantly higher for chordoma, highlighting different prognostic profiles between EWS and chordoma.

Pediatric Axial Ewing Sarcoma: A Retrospective Population-Based Survival Analysis.

Ewing sarcomas of the axial skeleton represent a notable challenge for clinicians because of their aggressive presentation and tendency to obstruct neurovascular structures; however, little data exist regarding axial tumors in children. This study is the first population-based analysis assessing treatment regimens for axial Ewing sarcomas and their effects on cancer-specific survival and overall survival (OS). Data from 2004 to 2019 were collected for all patients aged 1 to 24 years from the Surveillance, Epidemiology, and End Results (SEER) database. Primary groups included pelvic tumors, thoracic tumors, and vertebral tumors. Chi-squared and Kaplan-Meier tests were used to assess associations between demographic variables, clinical and treatment characteristics, and patient survival. Pelvic tumors were most common, and 49.7% received chemotherapy/radiation. Vertebral tumors were least common, and 56.7% received chemotherapy/surgery/radiation. 53.5% of thoracic tumors received chemotherapy/surgery. Surgery was most common for thoracic tumors (80.2%) and rare for pelvic tumors (38.9%). Radiation therapy was most common for vertebral tumors (83.6%) and least common for thoracic tumors (36.0%). Pelvic tumors exhibited the lowest OS (1-year, 5-year, and 10-year OS: 96%, 70%, and 59%), followed by thoracic tumors (1-year, 5-year, and 10-year OS: 97%, 79%, and 66%) and vertebral tumors (1-year, 5-year, and 10-year OS: 92%, 77%, and 68%). This study underpins the importance of both early detection and chemotherapy-based multimodal therapy in the treatment of axial Ewing sarcoma in a pediatric population. A comparatively large decline in OS was observed between 5 and 10 years for patients with thoracic tumors, and this cohort's 10-year OS has not improved when compared with a similar SEER cohort from 1973 to 2011. Despite a growing body of research supporting definitive radiation therapy, a notable portion of patients with pelvic Ewing sarcoma did not receive radiation, representing an unmet need for this population.

Outcomes of ilium and iliosacral Ewing's sarcoma resection reconstructed with tibial strut allograft.

Ilium is the most common site of pelvic Ewing's sarcoma (ES). Resection of the ilium and iliosacral joint causes pelvic disruption. However, the outcomes of resection and reconstruction are not well described. In this study, we report patients' outcomes after resection of the ilium and iliosacral ES and reconstruction with a tibial strut allograft. Medical files of 43 patients with ilium and iliosacral ES who underwent surgical resection and reconstruction with a tibial strut allograft between January 2010 and October 2021 were reviewed. The lesions were classified into four resection zones: I1, I2, I3, and I4, based on the extent of resection. Functional outcomes, oncological outcomes, and surgical complications for each resection zone were of interest. Functional outcomes were assessed using a Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score (TESS). The mean age of the patients was 17 years (SD 9.1). At a mean follow-up of 70.8 months (SD 50), the mean functional outcomes were 24.2 points (SD 6.3) for MSTS and 81 points (SD 11) for TESS. The mean MSTS and TESS scores were associated with the iliac resection zone (< 0.001). Nine patients (20.9%) had local recurrence. The recurrence was not associated with the zone of iliac resection (p = 0.324). The two-year disease-free survival of the patients was 69.4%. The mean time to graft union was longer in patients with the I4 resection zone (p < 0.001). The complication rate was 34.9%, and nerve palsy (11.6%) was the most common. The rate of surgical complications was not associated with the resection zone. Reconstruction using tibial strut allograft is an efficient procedure after the resection of the ilium and iliosacral ES. Functional outcomes and complications of iliac ES depend on the resection zone, and inferior outcomes could be generally expected when more segments of the pelvic ring are resected, even if it is reconstructed.

An unusual cystic presentation of pelvic skeletal Ewing sarcoma: a case series.

Ewing sarcoma (ES) is the second mostcommon primarymalignant bone tumour in children and adolescents. About 14.5% of primary malignancies develop in pelvic bones, wherethey typically have worse prognoses than extremity or acral sarcomas. It usually presents with aggressive features onradiology scans, but may also present with different radiological characteristics. In this series, we describe rare appearancesof pelvic skeletal Ewing sarcoma, with large extraosseous cystic component on imaging, defined by the presence of fluid-filledspaces in the extraosseous tumour lesion, which distinguishes it from the solid nature of conventional ES. We report3 cases of cystic presentation of ES, with imaging features supporting diagnosis of a primary malignant bone tumourarising from the superior pubic ramus with associated massive intrapelvic solid and cystic mass. CT-guided biopsy provideddiagnosis of ES, with large intrapelvic soft tissue and cystic component. These patients underwent neo-adjuvantchemotherapy and proton beam therapy with significant reduction in size of the solid components, while the cysticcomponents remained relatively unchanged. Two patients underwent surgical resection of the tumour (navigated P3internal hemipelvectomy and hemipelvis P2/P3 resection, respectively), and one patient died while on treatment. In both whounderwent surgery, histology showed ES with margins clear and more than 99% of treatment-induced necrosis. To theauthors' knowledge, this unusual presentation of pelvic ES is described for the first time in the literature as a case series,with particular reference to atypical extraosseous cystic changes, along with the clinical and radiological characteristics,and their treatment.

Complete pathological response of pelvic Ewing sarcoma in adult female—a case report

Complete pathological response of pelvic Ewing sarcoma in adult female—a case report

Adamantinoma like Ewing sarcoma of pelvis- A rare tumor at an uncommon site

Adamantinoma like Ewing sarcoma of pelvis- A rare tumor at an uncommon site

Pelvic Ewing sarcoma in children: Multimodal treatment, long term outcome and prognostic factors.

e22010 Background: The pelvis is one of the primary sites of Ewing sarcoma (ES) and is associated with a higher rate of local recurrence and poorer prognosis, compared with nonpelvic sites. Due to the rarity of this disease and limited data available, the prognostic factors of pelvic ES remain controversial. This study aims to assess the demographic, clinical features, treatment approach, outcome and prognostic factors in children with pelvic Ewing Sarcoma. Methods: Files of 264 children and adolescents with Ewing sarcoma diagnosed and treated between 1990-2022 in our center were retrospectively reviewed, of these 42 (15.9 %) were located in the pelvis. All recieved a COG modified chemotherapy regimen of ifosfamide-etoposide, alternating with vincristin, adriamisin/actinomycinD and cyclophosphamide (IE/VAC) every three weeks (every two weeks since 2012). Local treatment was discussed in each patient in the tumor board. Variables including age, gender, tumor stage, local and systemic treatment, outcome were analyzed using the Kaplan-Meier method and log rank test. Results: The median age of the 42 (23 male, 19 female) patients was 12.5 (2-16) years. The median time to diagnosis was 4 (0.25-12) months. The primary site was skeletal in 37 and extraskeletal in 5 patients. Seventeen (%40) had metastasis at diagnosis (lung, bone, bone marrow). Radiotherapy (RT) was given to 36 patients (10 preoperatively, 5 postoperatively, 21 definitive RT), 19 underwent surgery (4 at diagnosis, 14 had additional RT ). Surgical margins were positive in 6 and negative in 13. The histopathological necrosis was &lt;90% in 6 and ≥ 90% in 9. The median follow-up was 56 (10-370) months. At a median of 13 months (3-115), 22 patients had recurrence(9) /progression (13). The 3-year event free survival (EFS) and overall survival (OS) were 50.1% and 62.6%; in all; 62.2 and 75.3% in nonmetastatic; 31.9% ve %42.9% in metastatic patients ( p=0.048, p= 0.028) respectively. The 3-year EFS and OS were significantly higher in patients with negative surgical margins compared to ones with positive margins [(%83.9 vs 0 % (p=0.0001) and %91.7 vs %16.7 (p=0.001) respectively] . The 3-year EFS and overall survival OS were higher in patients with ≥90% necrosis [76.2 vs 33.3 % ( p=0.029); 87.5 vs 50% (p=0.13)respectively]. Age, gender, the mode of local therapy (surgery/RT/RT+surgery) was not prognostic. One patient who had recieved oral etoposide and RT at recurrence and who achieved a complete response, developed acute myeloid leukemia (AML), M5 myelomonocytic subtype, 20 months after relapse, and died. Conclusions: In our cohort of patients with pelvic Ewing sarcoma, 40% had metastasis at diagnosis. Metastasis, positive surgical margins, poor necrosois rate &lt;90% after neoadjuvant chemotherapy were found to be poor prognostic factors for outcome. Patients with localized pelvic Ewing sarcoma had a successful outcome with multidisciplinary treatment approach.

Particle Therapy in Adult Patients with Pelvic Ewing Sarcoma-Tumor and Treatment Characteristics and Early Clinical Outcomes.

To report dosimetric characteristics and early clinical outcomes in patients with pelvic Ewing sarcoma undergoing particle therapy. Patients ≥ 18 years old with pelvic Ewing sarcoma treated in adjuvant or definitive settings were considered for this retrospective analysis. Proton therapy was carried out with 45-60 Gy (RBE) (1.5-2 Gy (RBE) per fraction) and carbon ion therapy for recurrent disease with 51 Gy (RBE) (3 Gy (RBE) per fraction). Local control (LC), disease control (DC) and overall survival (OS) were calculated using the Kaplan-Meier method. For our sample, 21 patients were available, 18 of whom were treated for primary, 3 for locally recurrent and 16 for inoperable disease. The median CTV and PTV were 1215 cm3 and 1630 cm3. Median Dmean values for the PTV, bladder and rectum and median V40 Gy for the bowel for patients undergoing proton therapy were 56 Gy (RBE), 0.6 Gy (RBE), 9 Gy (RBE) and 15 cm3, respectively. At the end of particle therapy, G 1-2 skin reactions (n = 16/21) and fatigue (n = 9/21) were the main reported symptoms. After a median follow-up of 21 months, the 2-year LC, DC and OS were 76%, 56% and 86%, respectively. Particle therapy in adult pelvic Ewing sarcoma is feasible and provides excellent dosimetric results. First clinical outcomes are promising; however, further long-term follow-up is needed.

What's New in Musculoskeletal Tumor Surgery.

What's New in Musculoskeletal Tumor Surgery.

Analysis of Local Control Outcomes and Clinical Prognostic Factors in Localized Pelvic Ewing Sarcoma Patients Treated with Radiation Therapy: A Report from the Children's Oncology Group

<h3>Purpose/Objective(s)</h3> To identify potential clinical prognostic factors associated with a higher risk of local recurrence in localized pelvic Ewing Sarcoma (ES) patients treated with radiation therapy. <h3>Materials/Methods</h3> Data for 101 patients treated with definitive radiotherapy (RT) or both surgery and radiation (S+RT) on INT-0091, INT-0154, and AEWS0031 were analyzed. Radiation and imaging data was reviewed at IROC QARC. Imaging data for patients who did not receive radiation was not available, so surgery only patients were excluded. Tumor size was classified as </≥ 8 cm in maximum dimension and </≥ 200 mL volumetrically. Pelvic subsites were categorized as sacrum, iliac/buttock, or ischiopubic-acetabulum. Five-year cumulative incidence of local failure was defined as standard. <h3>Results</h3> The most common primary pelvic subsite was sacrum, 44.6%. Median tumor size at diagnosis was 9.7 cm (range: 2.4-16.0 cm) in maximum dimension and 215.9 mL (range: 3.7-1400.0 mL) volumetrically. RT was utilized in 68% of patients and S+RT in 32%. There was no statistically significant difference in local therapy modality employed based on tumor subsite or size. The five-year cumulative incidence of local failure for the entire cohort was 19.0% (95% CI, 12.7-28.5%). RT was associated with a higher incidence of local failure at 25.0% compared to 6.3% for S+RT (p=0.046). There was no difference in local failure outcomes by tumor size </≥ 8 cm in maximum dimension or </≥ 200 mL volumetrically. A higher incidence of local failure was seen with each additional 100 mL of tumor at diagnosis for all patients (p=0.04) and for RT only patients (p=0.005). Tumors originating in the ischiopubic-acetabular region were associated with the highest local failure incidence of 37.5%, compared to 20.0% for iliac-buttock and 11.4% for sacrum (p=0.02). Ischiopubic-acetabulum tumors treated with RT alone had the highest local failure rate in the cohort (50.0%, p=0.06). Multivariable analysis demonstrated RT alone (HR 5.1, p=0.04), tumor subsite (particularly ischiopubic-acetabulum tumors, HR 4.6, p=0.02), and increasing volume per 100 mL (HR 1.2, p=0.01) were associated with a higher incidence of local recurrence. <h3>Conclusion</h3> Patients with pelvic ES selected for S+RT had lower rates of local failure compared to patients selected for definitive RT. Tumors involving the ischiopubic-acetabular region and increasing tumor volume at diagnosis likely correlate with inferior local tumor control outcomes, especially for RT only patients, indicating local therapy intensification may be warranted for this cohort. Tumor characteristics did not correlate with choice of local therapy modality suggesting an opportunity to develop best local therapy practices guidelines for future studies based on tumor features.

Analysis of Local Control Outcomes and Clinical Prognostic Factors in Localized Pelvic Ewing Sarcoma Patients Treated With Radiation Therapy: A Report From the Children's Oncology Group

To identify potential clinical prognostic factors associated with a higher risk of local recurrence in patients with localized pelvic Ewing sarcoma treated with radiation therapy. Data for 101 patients treated with definitive radiation therapy (RT) or both surgery and radiation (S+RT) to primary pelvic tumors on INT-0091, INT-0154, and AEWS0031 were analyzed. Imaging data for patients who did not receive radiation were not available for central review; therefore, patients with surgery alone were not included. Cumulative incidence rates for local failure at 5 years from time of local control were calculated accounting for competing risks. The most common pelvic subsite was sacrum (44.6%). RT was used in 68% of patients and S+RT in 32%. The local failure rate was 25.0% for RT and 6.3% for S+RT (P=.046). There was no statistically significant difference in local control modality by tumor characteristics. Tumors originating in the ischiopubic-acetabulum region were associated with the highest local failure incidence, 37.5% (P=.02, vs sacrum and iliac/buttock tumors), particularly those treated with RT (50.0%, P=.06). A higher incidence of local failure was seen with each additional 100 mL of tumor at diagnosis (P=.04). Multivariable analysis demonstrated RT alone (hazard ratio [HR], 5.1; P=.04), tumor subsite (particularly ischiopubic-acetabulum tumors; HR 4.6; P=.02), and increasing volume per 100 mL (HR, 1.2; P=.01) were associated with a higher incidence of local recurrence. Combination surgery and RT is associated with improved local control in patients with pelvic Ewing sarcoma compared with definitive RT. Tumors involving the ischiopubic-acetabulum region and increasing tumor volume at diagnosis are associated with inferior local control. Tumor characteristics did not correlate with choice of local therapy modality suggesting an opportunity to develop best local therapy practices guidelines for future studies based on tumor features.

Ewing sarcoma of the pelvis: Clinical features and overall survival,

IntroductionPrimary Ewing Sarcoma of Bone is a malignancy whose treatment requires both systemic chemotherapy and local control through surgical resection and/or radiation. Ewing Sarcoma of the pelvis has been noted to confer a worse prognosis relative to other anatomic sites of Ewing Sarcoma. This study explores the presenting features, treatment modalities for local control, and overall survival of primary Ewing sarcoma of the pelvis in comparison to other commonly affected anatomic sites. MethodsThe National Cancer Institute Surveillance, Epidemiology, and End-Results (SEER) database was used to identify cases of pelvic Ewing sarcoma diagnosed between years 2004 and 2015. Demographic variables including sex, race, and age at diagnosis were described for each case, as well as therapeutic modalities including surgery and radiation. Bone-specific Collaborative Staging variables, including tumor size, tumor extension, and metastasis at diagnosis, were described for the same cohort. Univariate and multivariate assessments were performed for statistical comparison between presenting factors, treatment modalities, and between anatomic locations of presentation. ResultsWithin the database, 296 patients with Ewing sarcoma of the pelvic bones were available for review, which represented 25.7% of the 1152 cases surveyed across all anatomic sites. In the subset of patients with Ewing Sarcoma of the pelvis, 63.5% were male; the median age of diagnosis was 17 years; extra-compartmental tumor extension was noted in 82.1%; average tumor size was 9.7 cm; and metastasis at diagnosis was noted in 46.1% of the cohort. Only 28.6% of the pelvis sarcoma patients received surgical resection as part or all of their local control treatment, while 67.6% received some form of radiation therapy. When compared to the presenting features of Ewing Sarcoma from other anatomic sites, patients with pelvic tumors had larger tumors at time of diagnosis, higher rates of metastatic disease, and were less likely to undergo surgical intervention. The 2-, 5-, and 10-year overall survival rates for the patients presenting with Ewing Sarcoma of the pelvis was 70.3%, 49.7%, and 41.9%, respectively, which were significantly lower across all time-points than any other anatomic site. Discussion and ConclusionEwing Sarcoma of the pelvis is an aggressive malignancy that presents with relatively large tumors and a high rate of metastatic dissemination. Surgical intervention is less frequent when Ewing Sarcoma presents in the pelvis than when it presents in other anatomic locations. These factors may contribute to the worse overall survival of Ewing Sarcoma when compared to the same diagnosis originating in other anatomic sites. Prospective, randomized study is required to determine the true causal effects of these factors on survival.

Pelvic Ewing sarcoma: Should all patients receive pre-operative radiotherapy, or should it be delivered selectively?

BackgroundLocally recurrent disease following surgical resection of Ewing sarcoma (ES) confers a poor prognosis. Limited evidence is available evaluating non-selective use of pre-operative radiotherapy (RT) for patients with pelvic ES and its effect on local control and survival. Patients and methods49 consecutive patients with pelvic ES were identified retrospectively from a prospectively collated database. Patients either received non-selective pre-operative RT and surgery (n = 27), or selective post-operative RT (n = 22) (surgery alone (n = 11) or surgery and post-operative RT (n = 11)). ResultsPatients who had non-selective pre-operative radiotherapy appeared to have a higher LRFS, 88.0% compared to 66.5% in the selective RT group (p = 0.096, Kaplan Meier; p = 0.028, Chi-squared). Administration of non-selective, pre-operative RT to all patients with pelvic ES elevates the LFRS to that of the good responder group (≥90% tumour necrosis and margins, p = 0.880). There was no difference in metastasis-free survival, 60.0% and 54.5% (p = 0.728) and overall survival (OS), 57.7% and 63.6% (p = 0.893). The majority of pre-operative RT patients had both good necrosis (≥90%) (p = 0.003) and widely excised tumours, 81.5% vs 59.1% (p = 0.080). Tumour volume ≥250 ml was associated with worse LRFS (p = 0.045) and post-operative complications (p = 0.017). There may be improved LRFS (p = 0.057) with pre-operative proton-beam RT compared to surgery and selective post-operative RT. ConclusionPre-operative photon or proton-beam RT to all pelvic ES may improve LRFS compared to the selective delivery of post-operative RT. Radiotherapy delivered to all patients results in a greater percentage of highly necrotic tumours at surgical excision, enabling a greater proportion of patients with wide resection margins.

Pelvic Ring Reconstruction After Iliac or Iliosacral Resection of Pediatric Pelvic Ewing Sarcoma: Use of a Double-Barreled Free Vascularized Fibular Graft and Minimal Spinal Instrumentation.

In patients undergoing iliosacral resections, pelvic ring reconstruction can maintain stability of the pelvis and spinal column, which is expected to achieve good functional outcomes. However, no optimal reconstruction method has been established. We aimed to analyze the outcome of pelvic ring reconstruction using double-barreled free vascularized fibular graft (FVFG) and internal fixation after iliosacral resections in children. We retrospectively reviewed 16 children with pelvic Ewing sarcoma who underwent pelvic ring reconstruction using double-barreled FVFG after iliosacral resection. The fibular graft was placed between the supraacetabular region distally and the remaining ilium or sacrum proximally. The stability of the remaining pelvis and spinal column was provided by minimal spinal instrumentation. Eleven Type-I and 5 Type-I+IV resections were performed for 10 boys and 6 girls, who had a mean age of 13.4 years (range, 10 to 18 years). The mean follow-up was 49.8 months (range, 28 to 96 months). At the time of the final follow-up, 14 patients were alive and 2 patients had died of disease. The mean time for bone union was 9 months (range, 6 to 12 months). Graft hypertrophy was evident in all patients at 12 months. The median Musculoskeletal Tumor Society (MSTS) score at the time of the final follow-up was 80% (range, 60% to 96.6%). Seven patients had complications. Three complications required reoperation: 1 deep infection, 1 hematoma, and 1 wound dehiscence. Three patients had disease relapse in terms of lung metastases. This reconstruction method can achieve a high rate of bone union and can provide good functional outcomes following resection of pediatric pelvic Ewing sarcomas with iliosacral involvement. Complications are usually manageable without a need for revision surgical procedures. Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

A case of large pelvic Ewing sarcoma family of tumors successfully treated with high-dose chemotherapy and radiotherapy without surgical resection

A case of large pelvic Ewing sarcoma family of tumors successfully treated with high-dose chemotherapy and radiotherapy without surgical resection

Pelvic Ewing sarcoma: a retrospective outcome analysis of 104 patients who underwent pelvic tumor resection at a single supra-regional center

BackgroundLocal treatment in pelvic Ewing sarcoma (ES) consists of operation, radiation therapy, or a combination of both. Reported outcomes vary depending on the treatment modality performed. It is the objective of this study to analyze surgical outcome and complications as well as oncological outcome and complications of chemo- and radiation therapy in this patient cohort and evaluate prognostic factors.MethodsRetrospective review of 104 patients who underwent tumor resection for pelvic ES from 1988 to 2014.ResultsAll patients underwent pelvic resection and radiation therapy was administered in 77.9%. Margins were clear in 94.2%. The response to chemotherapy was good in 78.8%. Local recurrence occurred in 7.7%. The presence of distant metastases at the time of operation was the most important negative predictor for overall survival (p = 0.003). The cumulative 5- and 10-year survival rates were 82.7% and 80.1% for non-metastasized and 61.4% and 41.6% for metastasized pelvic ES at operation. In the presence of a single-distant metastatic site at operation compared to multiple metastatic sites, the cumulative survival rates were 64.3% versus 50% at five and 50.7% versus 16.7% at 10 years.ConclusionsA combined treatment approach of tumor resection and radiation therapy leads to a local control and overall survival rates comparable with those of extremity locations in this study’s patient cohort with localized pelvic ES. Therefore, surgical tumor resection (combined with (neo-)adjuvant radiation therapy) in non-metastatic pelvic ES seems feasible. In metastatic patients, however, the significance of tumor resection as a part of local treatment remains less certain and improved outcomes of combined local treatment approaches need to be weighed against these patients’ prognosis and quality of life.

PO-1232: Patients with pelvic Ewing Sarcoma with metastases at time of diagnosis: a monocentric experience

PO-1232: Patients with pelvic Ewing Sarcoma with metastases at time of diagnosis: a monocentric experience

Comparison of local control and survival outcomes between surgical and non-surgical local therapy on pelvic Ewing's sarcoma patients: a meta-analysis.

BackgroundThe efficacy of surgical therapy to nonsurgical therapy is still a controversial topic in pelvic Ewing’s sarcoma (ES) management. We perform a systemic review and meta-analysis to compare the effect of local control (LC) and survival outcomes between surgical and nonsurgical local therapy on pelvic ES patients with systemic chemotherapy.MethodsPublished retrospective studies searched from PubMed, Embase, Cochrane and Web of Science databases that investigated the effects of surgical and nonsurgical local therapy on the LC and survival outcomes of patients with pelvic ES treated with chemotherapy were included in our study. Our primary outcome was the LC rate and progression-free survival (PFS) rate. The effect of confounders of extend of disease, surgical margin and chemotherapy respond on PFS was analyzed in subgroups.ResultsTen studies with 782 pelvic ES patients were included in our analysis. Surgical patients showed higher LC and PFS rate comparing to nonsurgical patients [LC: risk ratio (RR) 0.72, 95% CI: 0.52–1.00, P=0.05, I2=0%; PFS: RR 0.72, 95% CI: 0.61–0.86, P=0.000, I2=15%]. Localized patients showed higher PFS with surgical therapy than nonsurgical patients (RR 0.67, 95% CI: 0.51–0.88, P=0.003).Patients with adequate resection and good chemotherapy respond improved PFS comparing to nonsurgical patients (adequate resection vs. nonsurgical: RR 0.59, 95% CI: 0.46–0.76, P<0.001, I2=0%; good respond vs. nonsurgical: RR 0.56, 95% CI: 0.41–0.77, P<0.001, I2=21%). But patients with inadequate resection and poor chemotherapeutic respond shows no statistical different PFS comparing to nonsurgical patients (inadequate resection vs. nonsurgical: RR 1.11, 95% CI: 0.87–1.41, P=0.41, I2=0%; poor respond vs. nonsurgical: RR 1.17, 95% CI: 0.90–1.52, P=0.25, I2=0%).ConclusionsSurgical therapy is primarily recommended in localized, resectable, good chemotherapeutic respond pelvic ES. Inadequate resection and poor chemotherapeutic respond are negative prognostic factors in surgical patients and their surviving are not improved comparing with nonsurgical patients.Systematic review registrationPROSPERO CRD42020149224.

Treatment Outcomes After Proton Therapy for Ewing Sarcoma of the Pelvis

Ewing sarcoma of the pelvis is associated with inferior local control compared with those arising from other primary sites. Despite its increased use, outcome data for treatment with proton therapy remain limited. We report 3-year disease control and toxicity in pediatric patients treated with proton therapy. Thirty-five patients aged ≤21 years (median, 14 years) with nonmetastatic pelvic Ewing sarcoma received proton therapy and chemotherapy between 2010 and 2018. Overall survival and tumor control rates were calculated using the Kaplan-Meier method. A log-rank test assessed significance between strata of prognostic factors. Significant toxicity was reported per the Common Terminology Criteria for Adverse Events, version 4.0. Most patients received definitive radiation (n = 26; median dose 55.8 Gy relative biological effectiveness [RBE]; range, 54.0-64.8), 7 received preoperative radiation (50.4 Gy RBE), and 2 received postoperative radiation (45 Gy RBE and 54 Gy RBE). The median primary tumor size was 10.5 cm. With a median follow-up of 3 years (range, 0.3-9.0 years), the 3-year overall survival, progression-free survival, and local control rates were 83% (95% confidence interval [CI], 65%-93%), 64% (95% CI, 45%-79%), and 92% (95% CI, 74%-98%), respectively. There was no association between local control, progression-free survival, or overall survival and tumor size, patient age, radiation dose, or definitive versus pre-/postoperative radiation therapy. Median time to progression was 1 year (range, 0.1-1.9 years). All patients with large tumors (≥8 cm) who underwent definitive proton therapy with a higher dose (≥59.4 Gy RBE) remained free from tumor recurrence (n = 5). Five patients experienced grade ≥2 subacute/late toxicity, all of whom were treated with combined surgery and radiation. Definitive proton therapy offers local control comparable to photon therapy in pediatric patients with pelvic Ewing sarcoma. These data lend preliminary support to radiation dose escalation without significant toxicity, which may contribute to the favorable outcomes. Combined surgery and radiation therapy, particularly preoperative radiation, is associated with postoperative complications, but not survival, compared with radiation alone.

Treatment of pelvic Ewing's sarcoma: Pros and cons of chemotherapy plus definitive radiotherapy versus surgery.

The aim of this study was to compare the results of chemotherapy or combined chemotherapy-radiation therapy with surgical intervention following neodjuvant therapy in pelvic Ewing's sarcoma patients. The study population consisted of 39 patients with pelvic Ewing's sarcoma treated in our clinic between 1994 and 2014. Of these patients, 28 patients (11 boys and 17 girls; mean age: 19.57±6.8 years) were treated with chemotherapy and radiation therapy and the remaining 11 patients (9 boys and 2 girls; mean age: 18.64±8.1 years) patients underwent surgical intervention after neoadjuvant chemotherapy or chemotherapy plus radiation therapy. Internal hemipelvectomy was performed in 10 patients, and external hemipelvectomy was performed in one patient. Survival rates were compared between the surgical and non-surgical treatment groups. Predictive factors, such as treatment protocol (surgery, neoadjuvant chemotherapy, definitive radiotherapy), mass localisation, mass size, presence of metastasis at the time of diagnosis, and presence of late metastases were compared between the groups. The effects of each variable on survival were also examined. The overall 3- and 5-year survival rates of the 28 non-surgical patients were 41.4% and 26.1%, respectively, while those of the surgical patients were 53% and 35.4%, respectively (p=0.777). Large mass size, presence of metastasis at the time of diagnosis, and presence of late metastases were significantly associated with lower survival rates. The survival rates of the patients who underwent surgery were higher than those of non-surgical patients, although the difference was not statistically significant. Definitive radiation and chemotherapy would be preferable in selected cases, such as patients with sacral localisation, without surgical intervention. Level III, Therapeutic Study.